FAP and Anesthesia

People with familial amyloid polyneuropathy (FAP) are likely to undergo procedures that require some form of anesthesia, which can carry extra risks for these patients.

Here is some information about the different types of anesthesia, and potential complications for people with FAP.

What is anesthesia?

Anesthesia involves the use of medications to prevent or manage pain during surgical or other procedures. The four main types of anesthesia are general, regional, local, and sedation.

General anesthesia is the use of medications, given directly into a vein or as an inhaled gas, to make a patient lose consciousness. Medical staff monitor the patient’s vital signs, such as blood pressure, heart rate, oxygen levels, and breathing to ensure safety. General anesthesia is usually necessary for major or lengthy surgeries.

Regional anesthesia involves the use of injections around a cluster of nerves, or those of the spinal cord, to block pain signals over a large area. Its use can make an entire limb or the lower half of the body unresponsive to pain. Epidurals given during childbirth are an example of a regional anesthetic. People given regional anesthesia are awake and alert during the procedure.

For procedures on smaller areas, such as stitching a deep cut or for minor dental work, a local anesthetic is sufficient. Local anesthetics can be given as an injection, or as a spray or cream.

Sedation, which can be given as an injection or inhaled gas, is given for more minor surgeries or less complex procedures that don’t require general anesthesia but need more than local anesthesia to prevent pain. Sedation relaxes a patient, and depending on the strength used can make them drowsy, even to the point of falling asleep or not remembering the procedure. But it does not render a person unconscious.

When might FAP patients need anesthesia?

In addition to treatment for an accident or other unpredictable event, a FAP patient is likely to need some form of anesthesia for procedures specific to this disease.

Biopsies are commonly used to diagnose FAP, and patients are likely to be given some form of an anesthetic to make the procedure more comfortable.

Major surgeries related to the treatment of select FAP patients, such as a kidney or liver transplant, would require anesthesia.

What are the potential risks of anesthesia?

Complications that can arise with the use of anesthesia with a FAP patient are often related to peripheral or autonomic neuropathy.

Peripheral neuropathy affects the peripheral nervous system, or the nervous system found outside of the brain and spinal cord. It could work against the effectiveness of regional anesthesia.

Autonomic neuropathy affects the autonomic nervous system that controls many body functions, including heartbeat and digestion. Some types of general anesthesia, when combined with autonomic neuropathy, can cause blood vessels to dilate and lead to hypotension or low blood pressure. Certain types of anesthetics can also lead to an increase in the concentrations of potassium in the blood, which can ultimately lead to arrhythmias or irregular heartbeats.

FAP can also affect the heart directly. The changes that this disease causes in the heart can make it more difficult for it to pump blood, raising the possibility of serious complications in a patient under anesthesia.

What precautions should doctors take?

Doctors should perform extensive testing before any procedures, whenever possible. They should especially investigate the degree of cardiac and nervous system involvement.

During anesthesia, extra care and monitoring needs to be given a FAP patient, with particular attention to heart rhythm and blood pressure measures for any signs of arrhythmia or hypotension.

In patients with autonomic neuropathy, it could be hard for a specialist to tell how deeply a patient is anesthetized, due to a poor response to certain stimuli. In these cases, clinicians may monitor the level of unconsciousness by looking at signals from the brain using electroencephalography (EEG) or evoked-potentials.

The possibility of multi-organ involvement in FAP also risks complications, and decisions regarding anesthesia need to be evaluated on a case-by-case basis.

Last updated: Feb. 11, 2021

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